Pierre-marie-bamberger syndrome M89.49

Initial description

The syndrome was first described by Bamberger in 1889. In 1890 Pierre Marie described the symptom picture of hypertrophic osteoarthropathy.

Pierre-Marie-Bamberger syndrome is a syndromal disease in which swelling occurs in the diaphyseal region, preferably in the long tubular bones, and changes in the end joints of the fingers and toes. The disease belongs to the "precancerous diseases".

The syndrome occurs in approximately 5% of patients withnon-small cell lung cancer.

In 70 - 90 % a malignant disease is found to be the cause of Pierre-Marie-Bamberger syndrome.

It also occurs in the following diseases:

• severe lung diseases
• congenital or acquired heart defects
• Pulmonary metastases
• Cirrhosis of the liver
• Leukaemia
• M. Hodgkin
• inflammatory bowel disease
• Occupational (pneumatic hammer)
• for otherwise healthy persons

Chronic hypoxia leads to erythrocytosis with formation of drumstick fingers and toes and watch glass nails and also to swelling in the area of the diaphyses, preferably on the long tubular bones. There are also indications of a venous circulatory disorder.

The patients show the following symptoms:

• Drum flail fingers or toes
• Watch glass nails
• Joint pain (can affect all joints, preferably medium-sized joints)
• Swelling of the joints
• Soft tissue thickening in the acra area
• chronic proliferative subperiosteal osteitis
• occasional redness or livid discoloration of the nail bed

The diagnosis is made on the basis of an X-ray of the affected parts of the skeleton. Here a periosteal proliferation in the area of the diaphyses of the tubular bones is shown.

Polyarthritis

Treatment of the underlying disease. The symptoms can almost completely disappear after the end of therapy.

Drum flail fingers and watch glass nails precede a carcinoma by weeks, therefore the above-mentioned symptoms should always be considered as a malignoma. The same applies if glucocorticoids, non-steroidal anti-inflammatory drugs and immunosuppressive drugs are not responded to in cases of arthritis.

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2. Bosnjakovic-Büscher S (1983) X-ray diagnostics of skeletal diseases Part 5 Osteopathies 609-633
3. Hatir I et al (2015) Journal of Rheumatology 74: 728-730
4. Herold G et al (2018) Internal Medicine 151
5. Schmalzing M (2018) Journal of Rheumatology 77: 309-321